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pedia - SLE
med22
| Question | Answer |
|---|---|
| What is the definition of Systemic Lupus Erythematosus (SLE)? | An autoimmune disease characterized by immune dysregulation, leading to multi-system inflammation and organ damage. It is often called The Disease with a Thousand Faces. |
| What is the typical age range and sex distribution for SLE diagnosis? | Most common in young women (female to male ratio of 9 to 1) and most frequently diagnosed in the teenage years to young adulthood. |
| What is the minimum number of ACR criteria required for the classification of SLE? | 4 out of 11 criteria. |
| ACR Criterion 1: Malar Rash | Fixed erythema over the malar eminences, flat or raised, typically sparing the nasolabial folds. |
| ACR Criterion 2: Discoid Rash | Erythematous raised patches with adherent keratotic scaling and follicular plugging, which can lead to atrophic scarring. |
| ACR Criterion 3: Photosensitivity | Skin rash resulting from an unusual reaction to sunlight, either by patient history or observed by a physician. |
| ACR Criterion 4: Oral Ulcers | Oral or nasopharyngeal ulcers, which are typically painless. |
| ACR Criterion 5: Arthritis | Non-erosive arthritis involving two or more peripheral joints (manifested by swelling or effusion). |
| ACR Criterion 6: Serositis | Pleuritis (pleural rub or effusion) OR Pericarditis (ECG, rub, or effusion, or evidence of pericardial effusion). |
| ACR Criterion 7: Renal Disorder (The two qualifying findings) | Persistent proteinuria greater than 0.5 g/day OR the presence of cellular casts (red cell, hemoglobin, granular, tubular, or mixed). |
| ACR Criterion 8: Neurologic Disorder (The two qualifying findings) | Unexplained Seizures OR Psychosis (after excluding other causes like drugs or metabolic issues). |
| ACR Criterion 9: Hematologic Disorder (List two key qualifying findings) | Hemolytic anemia with reticulocytosis OR Leukopenia less than 4,000/mm3 OR Lymphopenia less than 1,500/mm3 OR Thrombocytopenia less than 100,000/mm3. |
| ACR Criterion 10: Immunologic Disorder (List the three primary antibody findings) | Anti-dsDNA antibody OR Anti-Sm antibody OR a positive Antiphospholipid antibody (aPL) test. |
| ACR Criterion 11: Antinuclear Antibody (ANA) | Positive ANA titer (in the absence of drugs known to induce a lupus-like syndrome). |
| What is the most common and often earliest sign of Lupus Nephritis? | Proteinuria. |
| What specific type of neuropsychiatric symptom can be seen in SLE, potentially affecting school performance? | Cognitive impairment (e.g., difficulty with memory or concentration). |
| What two complement factors are typically low during an active flare of SLE? | C3 and C4. |
| What is the significance of a positive Anti-dsDNA antibody test? | It is highly specific for SLE and is often associated with active Lupus Nephritis. |
| What are the two first-line medications for the initial therapy of most SLE patients? | Hydroxychloroquine and Corticosteroids (Prednisone). |
| What is the main long-term benefit and primary role of Hydroxychloroquine in SLE management? | To reduce flares, prevent long-term organ damage, and prolong survival. |
| What is the main treatment for severe organ-threatening disease (e.g., severe Lupus Nephritis)? | Immunosuppressive agents like Azathioprine or Cyclophosphamide (for induction/maintenance therapy). |
| What complication of SLE is highly associated with long-term corticosteroid use? | Avascular Necrosis (especially of the femoral head). |
| What two non-pharmacological measures are essential for all SLE patients? | Sun avoidance and use of broad-spectrum sunscreen. |
| What type of vaccines should be avoided in immunosuppressed SLE patients? | Live vaccines (e.g., MMR, Varicella, Rotavirus). |
| What is the most frequent musculoskeletal manifestation in SLE? | Arthritis (non-erosive and symmetrical), often involving the hands, wrists, and knees. |